4-<wbr/>Methylumbelliferyl Palmitate

4-Methylumbelliferyl Palmitate

CAT N°: 16089
Price:

116.00 98.60

Cholesterol ester storage disease and Wolman disease are recessive autosomal disorders caused by a deficiency in lysosomal acid lipase (LAL), also known as cholesteryl ester hydrolase.{26282,26283} 4-Methylumbelliferyl palmitate (4-MUP) is a fluorogenic substrate for lysosomal acid lypase (LAL).{26284} 4-MUP is cleaved by LAL to release the fluorescent moiety 4-MU. 4-MU fluorescence is pH-dependent with excitation maxima of 320 and 360 nm at low (1.97-6.72) and high (7.12-10.3) pH, respectively, and an emission maximum ranging from 445 to 455 nM, increasing as pH decreases.{27251} 4-MUP may also be cleaved by other acid lipases.{26285,26286} Recent advances allow the assessment of LAL activity in very small blood volumes using 4-MUP.{26282,26283}

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